Consensus Report by PALISI and PBMTC Joint Working Committees, Supportive Care Guidelines for Management of VOD in Children and Adolescents Part 1: Focus on Investigations, Prophylaxis and Specific Treatment.

Publication Type:

Journal Article

Source:

Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation (2017)

Abstract:

Veno occlusive disease (VOD) is a common and potentially fatal complication in children undergoing hematopoietic cell transplantation (HCT). It occurs in about a third of all patients undergoing transplant and is fatal in 50% of patients with severe disease. Early intervention and specific treatment with defibrotide is associated with improved outcomes. However, there is lack of supportive care guidelines for management of the multi-organ dysfunction seen in most cases. There is high variability in the management of VOD which may contribute to the increased morbidity and mortality. While there is ample research in the specific treatment of VOD, there is paucity of literature regarding the management of ascites, transfusions requirements, fluids and electrolyte dysfunction, delirium and investigations in children with VOD. The joint working committees of the Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) and the Pediatric Blood and Marrow transplantation Consortium (PBMTC) collaborated to develop a series of evidence-based, supportive care guidelines for management of VOD. The quality of evidence was rated and recommendations made using the GRADE criteria. This manuscript is part 1 of the series and focuses on the need to develop these guidelines, methodology used to establish the guidelines, and investigations needed for diagnosis, prophylaxis, and treatment of VOD in children.